Scoring systems used to predict morbidity in children with Kawasaki disease (KD) have been developed and validated in Asian populations. The objective of this study was to assess their utility in predicting the development of coronary artery dilation in children with KD in North America. This was a secondary analysis of a National Institutes of Health / National Heart, Lung, and Blood Institute (NIH/NHLBI) Pediatric Heart Network public use dataset from a multicenter, randomized controlled trial of pulse steroid therapy in KD in a North American cohort. The primary outcome of interest was development of coronary artery dilation. The Harada, Kobayashi, Egami, and Sano scoring systems, originally developed to predict risk of intravenous immunoglobulin (IVIG) resistance in Kawasaki patients in Japan, were applied to this cohort. Subjects were kept in the analysis only if there were complete data for every element of each scoring system-Harada (n = 132), Kobayashi (n = 88), Egami (n = 139), and Sano (n = 82). Patients classified as high-risk by the Harada score were more likely to have significant coronary artery dilation (p = 0.042), were more likely to require IVIG retreatment (p = 0.002), and were more likely to require hospital readmission (p < 0.001). The Egami, Kobayashi, and Sano scores were not predictive for any measured outcome. The Harada score can be useful in identifying KD patients at risk for developing coronary artery dilation and IVIG resistance. The Harada score has demonstrated higher sensitivity but lower specificity, making it a valuable screening tool that may benefit from supplementary diagnostic methods.

UNASSIGNED: We aimed to explore simple and effective clinical parameters or combinations to predict coronary artery dilation and aneurysm formation in pediatric patients with Kawasaki disease (KD).
UNASSIGNED: This retrospective cohort study included pediatric patients with KD from January, 2013 to December, 2022. Multiple demographic and clinical data were collected, collated, and calculated from the medical records. Then they were divided into the coronary artery dilation and aneurysm formation group or the non-coronary artery dilation and aneurysm formation group. Lymphocyte-C-reactive protein ratio (LCR) was transformed into its natural logarithm and expressed as lnLCR.
UNASSIGNED: A total of 64 pediatric patients with KD were enrolled in this cohort study after 1:3 propensity score matching (PSM). For each unit increase in lnLCR, the possibility of coronary artery dilation and aneurysm formation decreased to 0.419 times the original value. The areas under the receiver operating characteristic (ROC) curves of lnLCR combined with albumin (ALB), ALB, and lnLCR to classify pediatric patients with KD into the coronary artery dilation and aneurysm formation group were 0.781, 0.692, and 0.743, respectively.
UNASSIGNED: LCR combined with ALB upon admission is a promising predictor of coronary artery dilation and aneurysm formation in pediatric patients with KD.

Geographical and racial factors constitute important distinctions between Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C), but no study has been conducted in Vietnam. Forty-one children with KD from January 2018 to July 2020 and 42 with KD/MIS-C from August 2020 to December 2022 were included in this study. Of the patients, 52.3% were aged between 12 and 35 months. Only two were aged over 5 years, and both were belong to the KD/MIS-C group. A 59.5% of the patients were male. Apart from fever, all symptoms tended to be more frequent in patients with KD/MIS-C. The prevalence of diffuse skin rash, hand and foot edema or erythema and gastrointestinal signs was significantly higher in patients hospitalized with KD/MIS-C. There was no significant difference in laboratory findings between the two groups. Coronary artery dilation was more frequently observed in patients with KD/MIS-C compared to those with KD (40.5% vs. 14.6%, P = 0.009).

BACKGROUND: Infection with Sars-CoV-2 is known to cause cardiac injury and coronary artery changes in moderate to severe acute COVID-19 and post-acute multisystem inflammatory syndrome in children (MIS-C). However, little is known about the potential for cardiac involvement, in particular coronary artery dilation, in asymptomatic or mild cases of COVID-19.
METHODS: A retrospective review of children ≤ 18 years of age with a history of asymptomatic or mild COVID-19 disease who underwent echocardiography after Sars-CoV-2 infection is conducted. Patients were excluded if they had been hospitalised for COVID-19/MIS-C or had a history of cardiac disease that could affect coronary artery dimension. Coronary artery dilation was defined as the Boston Z-score greater than 2.0.
RESULTS: One hundred and fifty-seven patients met inclusion criteria with a mean age of 9.4 years (+/- 5.4 years). Eighty-four (54%) patients were identified as having COVID-19 through positive antibody testing. All patients underwent electrocardiogram and echocardiogram as part of their cardiology evaluation. One hundred and thirty-five (86%) patients had a normal evaluation or only a minor variant on electrocardiogram, while 22 patients had abnormalities on echocardiogram, 4 of which demonstrated coronary artery dilation based on the Boston Z-score.
CONCLUSIONS: Much of the literature for post-infectious screening and follow-up focuses on patients with a history of moderate to severe COVID-19 disease, emphasising the need for surveillance for the potential development of myocarditis. In this study, 4 out of 157 (2.5%) children with a history of asymptomatic or mild COVID-19 disease without MIS-C were found to have some degree of coronary artery dilation. The significance of this finding currently remains unknown.

We reported a patient with a fistula of the right coronary artery to the left ventricle, accompanied by dilation of the right coronary artery and persistent chest pain. This patient underwent surgical fistula closure surgery, but the fistula recurred. Persistent chest pain reappeared after encountering COVID-19 infection. We analyzed the mechanism of persistent myocardial ischemic chest pain caused by coronary artery fistula in this patient, the impact of surgery on the patient\'s disease, the possible mechanism of COVID-19 causing persistent ischemic chest pain in this patient, and the possible mechanism of metoprolol in alleviating myocardial ischemic chest pain in this patient.

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Noonan Syndrome is caused by variants in a variety of genes found in the RAS/MAPK pathway. As more causative genes for Noonan Syndrome have been identified, more phenotype variability has been found, particularly congenital heart defects. Here, we report a case of dilated coronary arteries in a pediatric patient with a RIT1 variant to add to the body of literature around this rare presentation of Noonan Syndrome.  CASE PRESENTATION: A 2-month-old female was admitted due to increasing coronary artery dilation and elevated inflammatory markers. Rapid whole genome sequencing was performed and a likely pathogenic RIT1 variant was detected. This gene has been associated with a rare form of Noonan Syndrome and associated heart defects. Diagnosis of the RIT1 variant also gave reassurance about the patient\'s cardiac findings and allowed for more timely discharge as she was discharged to home the following day.  CONCLUSIONS: This case highlights the importance of the association between dilated coronary arteries and Noonan syndrome and that careful cardiac screening should be advised in patients diagnosed with Noonan syndrome. In addition, this case emphasizes the importance of involvement of other subspecialities to determine a diagnosis. Through multidisciplinary medicine, the patient was able to return home in a timely manner with a diagnosis and the reassurance that despite her dilated coronary arteries and elevated inflammatory markers there was no immediate concern to her health.

UNASSIGNED: As we learn more about the novel multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 infection, the protocols for long-term follow-up have evolved and only some of these protocols have been published. Here, we review the current literature on follow-up guidelines in MIS-C patients.
UNASSIGNED: We conducted a PUBMED search of all articles published on \"MIS-C\" and the term \"follow-up\" between 2020 and 2022. Inclusion criteria were that (1) the study was an observational study or case series, and (2) the study population included pediatric population who met the diagnostic criteria for MIS-C.
UNASSIGNED: There were 206 publications on MIS-C and follow-up in the last 2 years with 11 studies that fit the inclusion criteria. These papers were representing 11 different centers and encompassed a total of 343 participants. Seven of the 11 studies had participants follow-up with their cardiologist within 1 month of discharge. Between 12% and 62% of patients within each study had depressed left ventricular ejection fraction (LVEF) at admission. At the initial follow-up visit, five studies showed a normal LVEF in all patients while the other seven studies showed 2%-13% patients continuing to have depressed LVEF. In eight of the 11 studies, 9%-52% of patients had coronary artery dilation at admission. At their initial follow-up visit, 3%-28% of patients continued to have coronary artery dilation.
UNASSIGNED: There is some institutional variation in the outpatient follow-up protocols in patients diagnosed with MIS-C. A standardized follow-up guidelines might be helpful to monitor long-term prognosis of these patients.

BACKGROUND: Right coronary artery (RCA) fistulized to the coronary sinus is rare condition in adult cardiac anomalies, and the management and operative indication are controversial.
METHODS: We describe the case of a 45-year female patient who presented with exertional dyspnea, accompanied by intermitted lower limbs and facial edema. She was diagnosed with severe tricuspid regurgitation second to a severely dilated RCA fistulized to the coronary sinus. After multidisciplinary discussion, she underwent surgery through routine medium sternotomy, the right atrium was opened under cardiopulmonary bypass. The coronary arteriovenous fistula from the distal portion of RC to a severely enlarged coronary sinus was found. Trans-coronary sinus closure of the fistula was performed with continuous stitching and a tricuspid ring annuloplasty was done. The patient recovered uneventful post operation.
CONCLUSIONS: According to current literatures, surgical treatment was adopted for this case, instead of endovascular intervention. The optimal approach for these cases should consider the heart\'s anatomical characteristics. But we need to be aware of the occurrence of myocardial infarction and tricuspid regurgitation in the early and late stage after operation.

BACKGROUND: Epstein-Barr virus (EBV) infects more than 90% of the population worldwide. However, chronic active EBV infection (CAEBV) is one of the EBV-positive T- or NK-lymphoproliferative diseases with high morbidity and mortality. Here, we report a case of a 9-year girl with CAEBV, successively presenting with polymyositis and coronary artery dilation (CAD).
METHODS: The girl complained of fatigue for more than 1 month. Muscle strength examinations had no abnormal findings. Blood chemistries showed elevated alanine aminotransferase (ALT), aspartate aminotransferase (AST), and creatine kinase (CK). Magnetic resonance imaging (MRI) showed spotty high-intensity signals in thigh muscles, and electromyogram suggested myogenic damage. The significant findings were positive EBV antibodies (EBVEA-IgG, EBVCA-IgG, and EBVNA-IgG), increased EBV DNA copies in B, T, and NK cells, and positive EBV-encoded small RNA in biopsy muscle specimen. The girl received ganciclovir, intravenous immunoglobulin, and methylprednisolone, and her symptoms improved. On the 45th day of hospitalization, echocardiograph revealed CAD. She received additional anticoagulants and Tocilizumab. Her condition improved and continued to be followed up at the clinic preparing for hematopoietic stem cell transplantation.
CONCLUSIONS: This is the first reported case of CAEBV successively with polymyositis and CAD. This case makes the diagnoses of autoimmune diseases in children more complicated. Careful investigation of hidden CAEBV should be recommended in children with atypical polymyositis or CAD.